The Burden of Progressive Supranuclear Palsy on Patients, Caregivers, and Healthcare Systems by PSP Phenotype: A Cross-Sectional Study

Abstract

Progressive supranuclear palsy (PSP) is a rare, relentlessly progressive, ultimately fatal neurodegenerative brain disease. The objective of this study was to assess the burden of PSP on patients, caregivers, and healthcare systems by PSP phenotype. Data were drawn from the Adelphi PSP Disease Specific Programme™, a cross-sectional study of neurologists and people living with PSP in the United States of America, France, Germany, Italy, Spain, and the United Kingdom. All people living with PSP with a reported phenotype were included. PSP phenotype was reported for 242 patients (mean age: 70.2 years, 58% male): PSP-Richardson's syndrome, n = 96; PSP-predominant Parkinsonism, n = 88; PSP-predominant corticobasal syndrome, n = 28; PSP-predominant speech/language disorder, n = 12; PSP-progressive gait freezing, n = 9; PSP-predominant frontal presentation, n = 9. Most patients reported impaired cognitive, motor, behavioral and ocular functionality; 67–100% of patients (across phenotypes) had moderate-to-severe disease at the time of data collection. Post-diagnosis, the majority were provided with a visual and/or mobility aid (55–100%, across phenotypes), and/or required home modification to facilitate their needs (55–78%, across phenotypes). Patients required multiple types of healthcare professionals for disease management (mean 3.6–4.4, across phenotypes), and the majority reported receiving care from at least one caregiver (mean 1.3–1.8, across phenotypes). There is a high burden on patients, caregivers, and healthcare systems across all PSP phenotypes. Although phenotypes manifest different symptoms and are associated with different diagnostic pathways, once diagnosed with PSP, patients typically receive similar care.

Progressive Supranuclear palsy (PSP) disease progression, management, and healthcare resource utilization: a retrospective observational study in the US and Canada

“…Thus, some patients may continue on the drug postdiagnosis due to the potential for even a slight improvement, especially in the absence of a disease-modifying therapy. The PSP phenotype was not well documented in the records for most participants in the current study, suggesting that better education is required to make healthcare providers more aware of PSP phenotypes; however, it is likely that the majority of participants had the PSP-RS phenotype based on their reported symptoms and the fact that PSP-RS is the most common PSP phenotype [ 39 ].…”

“…Most participants in this cohort required some form of supportive care (86.1%), most commonly assistance from an unpaid caregiver while living at home (66.7%). A recent study reported a similar proportion of PSP patients with a caregiver (73%), but only 24% of the cohort used a professional caregiver [ 39 ]. The majority of PSP patient caregivers in other studies were family members [ 41 , 42 ], which is also consistent with our findings.…”

Patients with progressive supranuclear palsy need to be seen sooner and more frequently

“…Our recommendation is to create a care model whereby we treat atypical parkinsonism, not as a distant cousin of Parkinson’s disease, but proactively and with urgency, mirroring how our neuromuscular colleagues treat ALS. Indeed, the bulbar symptoms in atypical parkinsonism render their disease progression closer to ALS than to most cases of Parkinson’s disease after levodopa treatment [ 15 – 20 ].…”